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C1-抑制物和遗传性血管神经性水肿 被引量:3

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摘要 C1-抑制物(Cl-INH)是一种高度糖基化的血浆蛋白,属丝氨酸蛋白酶抑制剂超家族(Serpins superfamly),它在体内多个系统起重要的调节作用.Cl-INH先天性缺陷可导致发生遗传性血管神经性水肿(HAE).C1-INH基因组长约17kb,编码478个氨基酸的DNA序列长约1.8kb.IFN-γ,IL-6,IL-1和雄激素有增强Cl-INH基因表达的作用.HAE分为两型,其分子发病机制有所不同.治疗则多采用同化激素和Cl-INH浓缩制剂.
出处 《国外医学(免疫学分册)》 北大核心 1993年第1期1-5,共5页 Foreign Medical Sciences(Section of Immunology Foreign Medical Sciences)
关键词 C1-抑制物 水肿
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  • 1鹿育萨,李建民.丹那唑治疗遗传性血管性水肿的临床及C_1抑制物水平观察[J].山西医学院学报,1994,25(2):123-125. 被引量:1
  • 2任华丽,张宏誉.缓激肽与C_1抑制物缺乏症[J].医学综述,2005,11(6):491-493. 被引量:1
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